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抗α-氨基-3-羟基-5-甲基-4-异噁唑丙酸受体脑炎特征分析

Characteristic analysis of autoimmune encephalitis with antibodies against the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor

摘要回顾性收集2015年1月至2024年1月郑州大学第一附属医院收治的7例抗α-氨基-3-羟基-5-甲基-4-异噁唑丙酸受体(AMPAR)脑炎患者临床资料,并纳入87例资料完整文献病例汇总分析。7例患者年龄7~70岁,女性4例。7例患者均表现为边缘性脑炎,合并肺癌3例,胸腺瘤1例,均接受糖皮质激素和(或)丙种球蛋白治疗。随访1~25个月合并肿瘤的4例死亡。94例患者(7例收治病例+87例文献病例)合并肿瘤76例(80.9%),其中胸腺瘤33例(43.4%)、肺癌23例(30.3%)、乳腺癌9例(11.8%)及卵巢肿瘤6例(7.9%)。肿瘤诊断于脑炎症状前13例(18.8%),脑炎后1个月内40例(58.0%),1~6个月13例(18.8%),6~12个月2例(2.9%)及1年后1例(1.4%)。相比随访>1年未发现肿瘤的18例患者,合并肿瘤的76例患者年龄和精神异常比例高,而随访时间短,预后良好比例低(均 P<0.05)。抗AMPAR脑炎后1年内应积极随访,特别是肺、胸腺、乳腺和卵巢部位。合并肿瘤患者预后差,年龄较大及精神异常可能提示肿瘤的发生。

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abstractsThe clinical data of 7 patients with anti-α-amino-3-hydroxy-5-methyl-4- isoxazolepropionic acid receptor (AMPAR) encephalitis admitted to the First Affiliated Hospital of Zhengzhou University from January 2015 to January 2024 were retrospectively collected, and 87 cases with complete literature data were included for summary analysis. Seven casess exhibited limbic encephalitis, including 3 with lung cancer and 1 with thymoma. All cases were treated with glucocorticoids and/or human immunoglobulin. Four cases with concurrent tumors were followed up for 1 to 25 months and all died. Among the 94 patients (7 admitted cases and 87 documented cases), there were 76 cases with tumor (80.9%), including 33 cases of thymoma (43.4%), 23 cases of lung cancer (30.3%), 9 cases of breast cancer (11.8%), and 6 cases of ovarian tumor (7.9%). The tumor was diagnosed in 13 cases (18.8%) before encephalitis, 40 cases (58.0%) within 1 month after encephalitis, 13 cases (18.8%) within 1-6 months, 2 case (2.9%) within 6-12 months, and 1 case (1.4%) after 1 year. Compared to the 18 patients without tumors who were followed up for>1 year, the 76 patients with tumors showed an increase in age and incidence of mental disorders (both P<0.05), while follow-up time and proportion of good prognosis were decreased (both P<0.05). Closely follow-up should be conducted within 1 year after anti-AMPAR encephalitis, especially in the lungs, thymus, breast, and ovaries. The prognosis of patients with tumors is poor, and older age and mental disorders may indicate the occurrence of tumors.

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中华医学杂志

中华医学杂志

2024年104卷39期

3681-3684页

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